an introduction to cerebral palsy and hypoxic ischaemic encephalopathy

An introduction to Cerebral Palsy and Hypoxic- Ischaemic

Encephalopathy

5th Year Medical StudentImperial College London

1Imperial College London

Why Cerebral Palsy Cerebral Palsy (CP) is a common cause of

functional impairment in children that results in a permanent disability (1/500 live births(1))

The definitions and classifications of Cerebral Palsy can often be confusing

It is a condition that you are very likely to encounter during your career

An area often not covered at an undergraduate level....

But is a very examinable topic


Objectives Define cerebral palsy Describe the common causes of cerebral palsy Understand the different classifications of the

disease Have an understanding the multi-disciplinary

approach to its’ management Understand the prognosis and complications

associated with cerebral palsy Have a brief understanding of HIE and its’ role in

preventing cerebral palsy Understand the use of the APGAR scoring system To be able to answer exam questions on cerebral

palsy


Outline Cerebral Palsy

Definition and introductionPresentationCausesClassificationsManagementPrognosis

HIEOutline, Purpose, Future research

Conclusion Revision questions throughout


Definition“A disorder of movement and posture due to a non-

progressive lesion of the motor pathways in the developing brain”(2)

The disorder occurs whilst the brain is maturing, but the insult results in an injury that remains constant. The symptoms however may only develop as the child develops.

If the lesion is constant, why do the symptoms only develop as the child

develops?


Definition (2)A deficit in a certain area of the brain may only

become apparent once the child fails to reach a milestone involving that area of the brain (e.g if the lesion affects the child’s ability to walk, this may not become apparent in the first few months of life).

Also, the severity of the lesion becomes more apparent with time as the child falls further behind on reaching subsequent developmental milestones.


Presentation Motor disorder (positive or

negative) features with or without:- Learning difficulties (60%)- Epilepsy (40%)- Malnourishment (30%)- Visual Impairment- Auditory Impairment- Speech and Language Difficulties- Behavioural Problems- Contractures (1,3)


Positive featuresSpasticity, clonus, rigidity, spasms

Negative featuresWeakness, fatigue,Poor co-ordination

Pathophysiology Common misconception that cerebral palsy is usually

caused by problems (ie. hypoxia) at birth Up to 50% of children with CP have no identifiable cause/

origin (4)

Of those that can be, prenatal causes are the most common

Causes:

Birth

Antenatal period

Postnatal period

Hypoxic Ischaemic Encephalopathy

Periventricular LeucomalaciaIntraventricular HaemorrhageBoth assoc with prematurity.

Abnormal structural developmentCongenital infectionVascular occlusion (3)

80%

10% 10%


The biggest risk factor for the development of CP is prematurity . However, the majority of affected children are born at full term.

Classifications

Cerebral PalsySpastic 70%Hemiplegia

Diplegia

Quadraplegia

Ataxic Hypotonic 10%

Dyskinetic 10%

Mixed 10%

Cerebral Palsy is not just one disease, it can be classified according to its clinical features resulting from the area of brain that is affected.At risk babies are usually identified in the neonatal period as they fail to pass key milestones.

INJURY


Spastic: Hemiplegia Unilateral Upper limbs affected more than

lower Sign: Tip-toe walking on affected

side Also flexed, pronated forearm with

fisted hand Often no clear cause/ identifiable

event


Arms are affected more than legs as they have a larger representation within the motor cortex (1).

Spastic: Quadraplegia Bilateral, all 4 limbs

affected. Upper limbs affected

more than lower Signs: central

hypotonia, poor head control,

Most disabling form and these children require the most care (2)


Spastic Diplegia Bilateral, all four limbs

affected Legs affected much

more than arms (arm and hand function may be near normal)

Associated with prematurity (periventricular leukoplakia and Intraventricular haemorrhage)

Cognition often normal (the lesion is in the periventricular white matter and therefore spares the grey matter)


Ataxic Hypotonic Signs: poor balance,

ataxic gait, intention tremor, hypotonia (limb and trunk)

Symmetrical signs (i.e. both sides equally affected)

Pathology: Dysfunction in cerebellum or its connections


Saggital Section. Red outline indicates cerebellum (6)

Dyskinetic Intelligence often

normal Signs: dystonia,

chorea, athetosis, poor feeding

Pathology: dysfunction or damage to basal ganglia (exrapyramidal) and/or associated pathways


Coronal Section. Yellow arrow and box indicates basal ganglia (6)

Kernicterus due to hyperbilirubinaemia can result is Dyskinetc CP

Normal motor development

8-9 months 12 months

~6 months

1 ½ months

Good neck strengthCan hold own head

Sits without supportCan reach out with hands

CrawlingUses furniture to stand up

Wide based independent walking

Timeline adapted from Pathways Awareness Foundation (7)

Abnormal Signs3

months • Difficulty lifting head

6 months• Unable to lift head • Poor head control• Difficulty reaching out with arms• Poor central tone• Stiff Legs

9 months• Hand dominance• Difficulty crawling• Uses only one side of body to move• Inability to straighten back• Primitive Reflexes• Unable to weight bare

12 months• Difficulty in standing• Only uses arms to pull up to standing• Sits with weight on one side• Uses hand to balance sitting15Imperial College London

Investigations This is to establish the extent and severity of

the disorder and how the child is affected

Full history including obstetric and developmental history

Clinical assessment: serial developmental assessments

Biochemical: to rule out or uncover metabolic disorders

MRI: To visualise and assess the lesion EEG: focal abnormalities may suggest epilepsy

(8)

This provides a guide as to how the child is likely to progress in the future and the level of care that they are likely to need


Early Warning Signs of CPMaintenance of primitive reflexes (moro, stepping, etc)Developmental delayPoor feeding/suckingIrritabilityHand dominance before 2 yearsToe-walkingEpilepsyScissoring of legs (9)

ManagementPhysicalRehabilitationPhysical Therapy: Muscle strengthening, wheelchairs, temporary braces.Occupational TherapySpeech and Language Therapy (SALT)Psychological/ SocialSocial and leisure activities, specialist groupsfamily- centred services, counselling for child +/- parents.

Medical

EducationalEducating the child so that they understand their disorder.Educating parents so they know what they can expect/ will need to do in the future.Assessing the child’s educational needs.Informing teachers of the child’s requirements.

Orthopaedic, Respiratory, Neurology, Gastroenterology,Medications: Spasticity, EpilepsyImmunisations


A patient centered multidisciplinary approach addressing all aspects of the child’s life

Prognosis

A fact that parents want to know early on Not always easy to assess especially before 1 year

old Final neurological deficit may not become clear

until child/ child’s brain has fully developed Each child with CP is different and the best

prognosis is through effective treatment and supportive care for both the child and family

The type and severity of the disease as well as the feeding skills are the major determinants in life expectancy

Overall, only 50% of children with severe CP reach 20 years old (10)


Will he able to walk? How bad will it be when he grows up? Will he need to go to a specialist school?

Questions What are the 3 main types of Cerebral Palsy?

Spastic, Ataxic hypotonic and Dyskinetic

When is an insult that will lead to CP most likely to occur?

The antenatal period (during pregnancy)

Which disorder is most commonly associated with CP?

Learning disabilities


Questions At what age can a child normally to sit

unsupported?6 months

At what age can a child normally walk unsupported?

12 months

Is it abnormal if a baby is unable to lift their own head at 2 months?

No, it is however more of a concern if the child is unable to do so at 3 months.


Questions Which form of CP is associated with:

Intact cognition, severely affected leg function with near normal arm function?

Spastic Diplegic Cerebral Palsy

Poor balance, ataxic gate and hypotonia?Ataxic Hypotonic Cerebral Palsy

Rhesus disease of the new born?Dyskinetic Cerebral Palsy


Summary A non-progressive lesion of the motor

pathways in the developing brain 3 causes: prenatal, postnatal, time of

birth 3 main classifications: spastic,

dyskinetic, ataxic 3 sub-classifcations for spasticity: hemi,

di, and quadraplegia 3 common complications: learning

difficulties, epilepsy, feeding and nutritional problems

Multidisciplinary approach to treatment Prognosis is related to the severity of the

disorder


Hypoxic- Ischaemic Encephalopahy

(HIE)


Pathophysiology HIE is a serious problem for both preterm and term neonates

with the spectrum of injury ranging from neuronal injury to encephalopathy and death

Incidence = 3–9/1000 term infants Of these, it is estimated 33-50% have severe adverse outcomes or

die (1,2)

Injury is caused by a decrease in the amount of oxygen supplied to the infant’s brain close to the time of birth. The resulting poor perfusion causes a deprivation or glucose and oxygen supply. This leads to a cascade of metabolic derangements including lactic acidosis, ATP reserve depletion and free radical formation resulting in cell death and loss of neuronal function.

The consequent reperfusion extends the damage and leads to delayed cell death (1)

HIE is the cause of CP in 8-15% of children (11)

This presents with signs of foetal distress, acidosis and a low APGAR score when the baby is born 24Imperial College London

APGAR score


0 1 2

Colour Pale/blue Body pink, blue

peripheries

Pink

Pulse None <100bpm >100bpm

Reflex Irritability

None Grimace Cry/ Cough

Muscle tone

Flaccid Some limb flexion

Active, well flexed

Respiratory Rate

None Gasping/ Irregular

Regular stong cry

Score Result0-3 Very low4-6 Mildly low

7-10 Normal

Provides an immediate objective measurement of the condition of the new born babyA score out of 10 is taken at 1, 5 and 10 minutes of life. Additional scores are taken very 5 minutes if necessaryA low score on the one-minute test may show that the neonate requires medical attention but is not necessarily an indication that there will be long-term problemsA score <3 at ≥10 minutes indicates a risk of neurological damage (eg Cerebral Palsy)

Management Hypothermia has recently emerged to be a safe and

possibly effective intervention (3)

Hypothermia therapy can slow the cascade and preserve cellular function, helping to stop the series of events that occur after reperfusion following the initial asphyxial event

Reducing body temperature 3-5°C below the normal level leads to:- A decrease in brain energy utilisation- Reduction of infarct size- Amelioration of neuronal cell loss- Improves neurological outcome after asphyxia (2)

This is through suppression of excitatory amino acid accumulation and a decrease in both cytokine levels and blood brain barrier permeability


Hypothermia Therapy Once it has been established that the baby is at

risk of brain damage and fulfils entry criteria for hypothermia therapy, passive cooling is started

This involves turning off the resuscitaire heater and removing all clothing/ blankets from the baby to allow the body temperature to naturally come down

Cranial or full body hypothermia therapy should then be started within 6 hours of birth

Hypothermia therapy can either be full body or head selective (with the use of a purpose-made cap)


Research and Prognosis CoolCap, NICHD, TOBY studies looked at the use of

hypothermia therapy in HIE

Entry Criteria used in 3 main studies:- pH <7.0 or BE <-16 in first hour of life- Assisted ventilation by 10 min or 10min Apgar score ≤5- Abnormal neurological tests (aEEG) / Signs of foetal distress

Duration: commenced within 6hrs of birth for 72hrs, followed by re-warming at 0.5C/hr

Depth: Full body aim for 33.5°C (rectal), selective head aim for 34.5°C (rectal)

Result: Cooling significantly reduces the occurrence of poor outcome (death and disability) and increases the number survivors with normal neurological function at 18-month follow-up (4,5)


HIE summary HIE in the perinatal period is a major cause of

neonatal death and long-term disability.

Advances in research are uncovering the pathophysiology underlying HIE

HT is the most effective treatment currently (1) Further Reading

- A Parish and J Bhatia: Hypothermia for hypoxic-ischemic brain injury- M Thoresen: Hypothermia after Perinatal Asphyxia:- Selection for Treatment and Cooling Protocol- NICE Guidelines: Therapeutic hypothermia with intracorporealtemperature monitoring for hypoxic perinatal brain injury


Questions What is the APGAR score for the following babies?

a) HR= 125, pink peripheries, a cough when stimulated, some flexion and strong breathing?

9 b) HR= 55, grimace when stimulated, weak breathing, cyanosed

and some limb flexion? 4

c) Cyanosed, no breathing, no pulse, no flexion and no response on stimulation?

0


0 1 2

Colour Pale/blue

Body pink, blue

peripheries

Pink

Pulse None <100bpm >100bpm

Reflex Irritability

None Grimace Cry/ Cough

Muscle tone

Flaccid Some limb flexion

Active, good

flexionRespiratory Rate

None Gasping/ Irregular

Regular stong cry

Conclusion Cerebral Palsy is a permanent and

disabling disorder of motor function Cerebral Palsy is a multi-factorial

disease that requires multi-disciplinary approach to treatment

HIE is the most common cause of birth related CP

Hypothermia therapy is an effective and developing treatment in limiting/ preventing neonatal brain injury


Objectives Define cerebral palsy Describe the common causes of cerebral

palsy Understand the different classifications of

the disease Have an understanding the multi-

disciplinary approach to its’ management Understand the prognosis and

complications associated with cerebral palsy

Have a brief understanding of HIE and its’ role in preventing cerebral palsy

Understand the use of the APGAR scoring system

To be able to answer exam questions on cerebral palsy


Thank you


“You learn something new every day.....if you pay attention.”

My Dad

Cerebral Palsy References 1. Mohammed M.S. Cerebral Palsy: Comprehensive Review and Update. Ann

Saudi Med 2006; 26(2):123-132.

2. Shevell MI, Bodensteiner JB. Cerebral Palsy: Defining the problem. Semin Paed Neurology 2004;11(1):2-4

3. Lissauer T, Clayden G (ed). Illustrated Textbook of Paediatrics. 3rd Edition.Philadelphia: Mosby Elsevier.

4. Taft L. Cerebral Palsy. Paediatric Rev 1995;16:411-418

5. Matthews D, Wilson P. Cerebral Palsy. In: Molnar G, Alexander M (eds). Peadiatric Rehab 3rd Edition. Philadelphia: Hanley and Belfus, Inc; 1998:193.218.

6. Politis M. Basal Ganglia and Cerebellum [Lecture] 2010. Imperial College London, 27th October.

7. Pathways Awareness Foundation (nd). Monthly Milestones [Online]. Available from: http://www.pathwaysawareness.org/milestones [Accessed: June 4th 2011].

8. Jan MMS: Assessment of the utility of Paediatric Electroencephalography. Seizure 2002;11(2):9.103.

9. McMurray J, Wilson CD, Fahn S. Dopa. Responsive dystonia: long term treatment response and prognosis. Neurology 1991;41:174.181.

10. Hutton J, Cooke T, Pharoah P. Life expectancy in children with Cerebral Palsy. BMJ 1994;309:431.435

11. Pschirrer R, Yeomans E. Does asphyxia cause Cerebral Palsy? Seminar Perinatol 2000;24:215.220.


http://www.pathwaysawareness.org/milestones

HIE References 1.Parish A, Bhatia J. Hypothermia for hypoxic-ischemic brain injury. Journal of

Maternal-Fetal and Neonatal Medicine. 2009;22:719-721

2. Lai MC, Yang SN. Perinatal Hypoxic-Ischaemic Encephalopathy. Journal of Biomedicine and Biotechnolog. 2011

3. Vannucci RC, Perlman M. Interventions for perinatal hypoxic-ischemic encephalopathy. Peadiatrics 1997;100:1004-1014

4. Thoresen M. Hypothermia after Perinatal Asphyxia: Selection for Treatment and Cooling Protocol. 2010;158: Suppl 1 45-49.

5. 7. NICE: Therapeutic hypothermia with intracorporeal temperature monitoring for hypoxic perinatal brain injury. May 2010.

6. Edwards AD, Broklehurst P, Gunn AJ, Halliday H, Juszczack E, Iwata O, et al. Neurological outcome at 18months of age following moderate hypothermia in newborn infants with hypoxic ischaemic incephalopathy. BMJ 2010;340:c363

7. Wilikinson DJ. Cool heads: ethical issues associated with therapeutic hypothermia for newborns. Acta Paediatr 2009;98:217-220.


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